DefinitionCystic fibrosis (CF) is a genetic disease. CF occurs in 1 in 2500 births in Australia and is the most common genetic disease in Caucasian Australians. CF prevents the body from absorbing enough nutrients. This makes it difficult for people with CF to meet increased nutrient needs. As a result, people with CF may need to eat an enriched diet with more kilojoules and take extra vitamins and enzymes.

InformationMost people with CF are diagnosed by the age of 3. Doctors and dieticians closely follow them. The dietician handles the complex nutritional issues that come up in the care of these individuals.

CF affects the mucous-producing glands in the pancreas, lungs and intestines. It causes thick mucous to build up and clog the lungs. This can lead to life-threatening infections. The mucous can also block the pancreas. This is the gland that makes many of the hormones and enzymes needed for digestion of food.

The mucous build up can cause malabsorption of nutrients. This is when nutrients from foods are not absorbed, but are instead passed out in the stool. Because of this, people with CF must eat a lot more food to receive enough kilojoules and nutrients to maintain normal weight. Children with CF, whose bodies are using kilojoules and nutrients to grow, must sometimes consume up to six times as many kilojoules as a healthy child in order to grow properly.

Enzymes are proteins made in our bodies. They spark various reactions, including those involved in the breakdown of food. Many times, a person with CF, does not produce enough of a fat-digestive enzyme called lipase. They may need to take specially formulated enzyme supplements, with each meal, to aid in digestion.

Since people with CF cannot absorb salt from sweat, they need salt, which is sodium chloride, in larger amounts. This is especially true during hot weather when there is increased sweating. Drinking plenty of fluids is important to avoid dehydration, or low body fluid levels.

Higher amounts of vitamins and minerals may also be needed. Because the body cannot absorb many nutrients, a person with CF needs about twice the recommended Dietary Intake (RDI) for fat-soluble vitamins, which are vitamins A, D,E and K. Many times supplements are needed.

Cystic fibrosis:

www.cysticfibrosisaustralia.org.au

www.vg.edu.au/vdw/cflink.htm (lots of interesting links named)

Author: Reviewer: eknowhow Medical Review PanelEditor: Dr John Hearne

Last Updated: 20/09/2004ContributorsPotential conflict of interest information for reviewers available on request

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